A Pleuro-Peritoneal Communication through the Diaphragm affected with Lymphangioleiomyomatosis

• Published in: Internal Medicine Vol. 49; no. 5; pp. 439 - 445
• Main Authors: Takagi, Yumiko, Sato, Teruhiko, Morio, Yoshiteru, Kumasaka, Toshio, Mitani, Keiko, Miyamoto, Hideaki, Iwakami, Shin-ichiro, Kodama, Yuzo, Onuma, Emi, Seyama, Kuniaki, Takahashi, Kazuhisa
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2010
• Subjects: Adult; chylothorax; Chylothorax - etiology; chylous ascites; Chylous Ascites - etiology; Diaphragm; Female; Humans; lymphangiogenesis; lymphangioleiomyomatosis; Lymphangioleiomyomatosis - complications; Lymphangioleiomyomatosis - surgery; Lymphatic Vessels - diagnostic imaging; Lymphatic Vessels - physiopathology; Muscle Neoplasms - complications; Muscle Neoplasms - surgery; Peritoneal Cavity - diagnostic imaging; Peritoneal Cavity - physiopathology; Pleural Cavity - diagnostic imaging; Pleural Cavity - physiopathology; pleuro-peritoneal communication; Radionuclide Imaging
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.49.2714

A 30-year-old Japanese woman with lymphangioleiomyomatosis (LAM) developed a left chylothorax and chylous ascites. A pleuro-peritoneal communication was confirmed by a scintigram with 99mTc-labeled macroaggregated-albumin injected into the peritoneal cavity. Video-assisted thoracic surgery revealed a protruding papillary lesion on the left diaphragm. Chyle was oozing into the pleural cavity through this lesion. Histopathological analyses demonstrated that the protrusion was a diaphragmatic LAM lesion and that LAM-associated lymphangiogenesis enabled communication between the pleural and peritoneal cavities through lymphatic vessels. This case demonstrated a new mechanism for chylous pleural effusion in LAM and illustrates the significance of LAM-associated lymphangiogenesis.